Frontotemporal dementia and amyotrophic lateral sclerosis: Revisiting one of the first case reports with neuropathological examination

Nitrini, Ricardo

doi:10.1590/S1980-57642014DN81000013

ABSTRACT

The occurrence of dementia in amyotrophic lateral sclerosis (ALS) was only widely recognized in the late 20^th century. Hitherto, it was believed that dementia was a rare event due to the fortuitous association with other diseases. In 1924, Kostantin Nikolaevich Tretiakoff and Moacyr de Freitas Amorim reported a case of dementia with features of frontotemporal dementia (FTD) that preceded the motor signs of ALS. Neuropathological examination confirmed ALS and found no signs of other dementia-causing diseases. The authors hypothesized that dementia was part of ALS and recommended the search for signs of involvement of motor neurons in cases of dementia with an ill-defined clinical picture, a practice currently accepted in the investigation of cases of FTD. This was one of the first descriptions of dementia preceding the motor impairments of ALS and was published in Portuguese and French in Memórias do Hospício de Juquery.

Key words:
dementia; frontotemporal dementia; amyotrophic lateral sclerosis; neuropathology; motor neuron disease

RESUMO

A ocorrência de demência na esclerose lateral amiotrófica (ELA) somente foi amplamente reconhecida no final do século 20. Antes disso, acreditava-se que demência era ocorrência rara que se devia à associação fortuita de outra doença. Em 1924, Kostantin Nikolaevich Tretiakoff e Moacyr de Freitas Amorim relataram caso de demência com as características de demência frontotemporal (DFT) que precedeu os sinais motores da ELA. O exame neuropatológico confirmou ELA e não encontrou sinais de outras doenças que cursam com demência. Os autores do artigo aventaram que a demência fazia parte da ELA e recomendaram a investigação da presença de sinais de comprometimento dos neurônios motores em dementes com alterações cognitivas e comportamentais mal definidas, conduta atualmente aceita na investigação de casos de DFT. Esta foi uma das primeiras descrições de demência precedendo a síndrome motora da ELA e foi publicada em português e em francês em Memórias do Hospício de Juquery.

Palavras-chave:
demência; demência frontotemporal; esclerose lateral amiotrófica; neuropatologia; doença do neurônio motor

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REFERENCES

¹
Charcot JM. De la sclérose latérale amyotrophique. Progrès Médical 1874;2:326-341.
²
Wechsler IS, Davison C. Amyotrophic lateral sclerosis with mental symptoms. Arch Neurol Psychiatr 1932;27:859-880.
³
Morita K, Kaiya H, Ikeda T, Namba M. Presenile dementia combined with amyotrophy: a review of 34 Japanese cases. Arch Gerontol Geriatr 1987;6:263-277.
⁴
Marie P. Leçons sur les maladies de la moelle. Masson: Paris, 1892.
⁵
Hudson AJ. Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain 1981;104:217-247.
⁶
Neary D, Snowden JS, Mann DMA, Northen B, Goulding PJ, Macdermott N. Frontal lobe dementia and motor neuron disease. J Neurol Neurosurg Psychiatry 1990;53:23-32.
⁷
Hirano A, Krooth RS, Lessell S, Kurland LT. Parkinsonism-dementia complex, an endemic disease on the island of Guam. 1. Clinical features. Brain 1961;84:642-661.
⁸
Salazar AM, Masters CL, Gajdusek DC, Gibbs CJ. Syndromes of amyotrophic lateral sclerosis and dementia; relation to transmissible Creutzfeldt-Jakob disease. Ann Neurol 1983;14:17-26.
⁹
Mitsuyama Y, Takamiya S. Presenile dementia with motor neuron disease: a new entity? Arch Neurol 1979;36:592-593.
¹⁰
Bonduelle M. Amyotrophic lateral sclerosis. In Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology. Amsterdam: North-Holland, 1975; 22:281-338.
¹¹
Ferrer I, Roig C, Espino A, Peiro G, Guiu XM. Dementia o frontal lobe type and motor neuron disease: a Golgi study of the frontal cortex. J Neurol Neurosurg Psychiatry 1991:54:932-934.
¹²
Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial "Clinical limits of amyotrophic lateral sclerosis" workshop contributors. J Neurol Sci 1994;124(Suppl):96-107.
¹³
Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry 1994;57:416-418.
¹⁴
Zago S, Poletti B, Morelli C, Doretti A, Silani V. Amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD). Arch Ital Biol 2011; 149:39-56.
¹⁵
Tretiakoff C, Amorim MF. Um caso de esclerose lateral amyotrophica pseudo-polynevritica em uma alienada portadora de tuberculose intestinal. Memórias do Hospício de Juquery (São Paulo, Brazil) 1924;1:81-90.
¹⁶
Tretiakoff C, Amorim MF. Un cas de sclérose latérale amyotrophique pseudo-polynevritique, observée chez une alienée, atteinte de tubérculose intestinale. Memórias do Hospício de Juquery (São Paulo, Brazil) 1924;1:259-266.
¹⁷
Godoy JM, Skacel M, Balassiano SL, et al. Esclerose lateral amiotrófica variante distal dos membros inferiores apresentação de três casos. Arq Neuropsiquiatr 1991;49:321-325.
¹⁸
Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134:2456-2477.
¹⁹
Chen S, Sayana P, Zhang X, Weidong L. Genetics of amyotrophic lateral sclerosis: an update. Mol Neurodegener 2013;8:28. Published online 2013 August 13. doi: 10.1186/1750-1326-8-28
» https://doi.org/10.1186/1750-1326-8-28
²⁰
Ziegler LH. Psychotic and emotional phenomena associated with amyotrophic lateral sclerosis. Arch Neurol Psychiatr 1930;24:930-936.
²¹
van Bogaert L. Les troubles mentaux dans la sclérose laterale amyotrophique. Encéphale 1925;20:27-47.
²²
Uematsu S. Amyotrophic lateral sclerosis and its mental symptoms. Shindan To Chiryo 1935;22:838-844 (in Japanese).
²³
Gerber I, Naville F. Contribution a l'étude histologique de la sclérose laterale amyotrofique. Encéphale 1921;16:113-126.
²⁴
Knopman DS, Mastri AR, Frey WH, Sung JH, Rustan T. Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology 1990;40:251-256.
²⁵
Cairns N, Bigio EH, Mackenzie IRA, et al. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol 2007;114:5-22.
²⁶
Pacheco e Silva AC. Introducção. Memórias do Hospício de Juquery (São Paulo, Brazil) 1924;1:IX-XI.
²⁷
Lees AJ, Selikhova M, Andrade LA, Duyckaerts C. The Black Stuff and Konstantin Nikolaevich Tretiakoff. Mov Disord 2008;23:777-783.
²⁸
Andrade LAF, Selikhova M, Lees AJ. Konstantin N. Tretiakoff in Brazil a historical perspective and discussion of his contribution to Brazilian neuroscience. Arq Neuropsiquiatr 2009;67:322-327.

Publication Dates

Publication in this collection
Jan-Mar 2014

History

Received
15 Nov 2013
Accepted
06 Jan 2014

This is an open-access article distributed under the terms of the Creative Commons Attribution License

[1] ¹
Charcot JM. De la sclérose latérale amyotrophique. Progrès Médical 1874;2:326-341.

[2] ²
Wechsler IS, Davison C. Amyotrophic lateral sclerosis with mental symptoms. Arch Neurol Psychiatr 1932;27:859-880.

[3] ³
Morita K, Kaiya H, Ikeda T, Namba M. Presenile dementia combined with amyotrophy: a review of 34 Japanese cases. Arch Gerontol Geriatr 1987;6:263-277.

[4] ⁴
Marie P. Leçons sur les maladies de la moelle. Masson: Paris, 1892.

[5] ⁵
Hudson AJ. Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain 1981;104:217-247.

[6] ⁶
Neary D, Snowden JS, Mann DMA, Northen B, Goulding PJ, Macdermott N. Frontal lobe dementia and motor neuron disease. J Neurol Neurosurg Psychiatry 1990;53:23-32.

[7] ⁷
Hirano A, Krooth RS, Lessell S, Kurland LT. Parkinsonism-dementia complex, an endemic disease on the island of Guam. 1. Clinical features. Brain 1961;84:642-661.

[8] ⁸
Salazar AM, Masters CL, Gajdusek DC, Gibbs CJ. Syndromes of amyotrophic lateral sclerosis and dementia; relation to transmissible Creutzfeldt-Jakob disease. Ann Neurol 1983;14:17-26.

[9] ⁹
Mitsuyama Y, Takamiya S. Presenile dementia with motor neuron disease: a new entity? Arch Neurol 1979;36:592-593.

[10] ¹⁰
Bonduelle M. Amyotrophic lateral sclerosis. In Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology. Amsterdam: North-Holland, 1975; 22:281-338.

[11] ¹¹
Ferrer I, Roig C, Espino A, Peiro G, Guiu XM. Dementia o frontal lobe type and motor neuron disease: a Golgi study of the frontal cortex. J Neurol Neurosurg Psychiatry 1991:54:932-934.

[12] ¹²
Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial "Clinical limits of amyotrophic lateral sclerosis" workshop contributors. J Neurol Sci 1994;124(Suppl):96-107.

[13] ¹³
Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry 1994;57:416-418.

[14] ¹⁴
Zago S, Poletti B, Morelli C, Doretti A, Silani V. Amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD). Arch Ital Biol 2011; 149:39-56.

[15] ¹⁵
Tretiakoff C, Amorim MF. Um caso de esclerose lateral amyotrophica pseudo-polynevritica em uma alienada portadora de tuberculose intestinal. Memórias do Hospício de Juquery (São Paulo, Brazil) 1924;1:81-90.

[16] ¹⁶
Tretiakoff C, Amorim MF. Un cas de sclérose latérale amyotrophique pseudo-polynevritique, observée chez une alienée, atteinte de tubérculose intestinale. Memórias do Hospício de Juquery (São Paulo, Brazil) 1924;1:259-266.

[17] ¹⁷
Godoy JM, Skacel M, Balassiano SL, et al. Esclerose lateral amiotrófica variante distal dos membros inferiores apresentação de três casos. Arq Neuropsiquiatr 1991;49:321-325.

[18] ¹⁸
Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134:2456-2477.

[19] ¹⁹
Chen S, Sayana P, Zhang X, Weidong L. Genetics of amyotrophic lateral sclerosis: an update. Mol Neurodegener 2013;8:28. Published online 2013 August 13. doi: 10.1186/1750-1326-8-28
» https://doi.org/10.1186/1750-1326-8-28

[20] ²⁰
Ziegler LH. Psychotic and emotional phenomena associated with amyotrophic lateral sclerosis. Arch Neurol Psychiatr 1930;24:930-936.

[21] ²¹
van Bogaert L. Les troubles mentaux dans la sclérose laterale amyotrophique. Encéphale 1925;20:27-47.

[22] ²²
Uematsu S. Amyotrophic lateral sclerosis and its mental symptoms. Shindan To Chiryo 1935;22:838-844 (in Japanese).

[23] ²³
Gerber I, Naville F. Contribution a l'étude histologique de la sclérose laterale amyotrofique. Encéphale 1921;16:113-126.

[24] ²⁴
Knopman DS, Mastri AR, Frey WH, Sung JH, Rustan T. Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology 1990;40:251-256.

[25] ²⁵
Cairns N, Bigio EH, Mackenzie IRA, et al. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol 2007;114:5-22.

[26] ²⁶
Pacheco e Silva AC. Introducção. Memórias do Hospício de Juquery (São Paulo, Brazil) 1924;1:IX-XI.

[27] ²⁷
Lees AJ, Selikhova M, Andrade LA, Duyckaerts C. The Black Stuff and Konstantin Nikolaevich Tretiakoff. Mov Disord 2008;23:777-783.

[28] ²⁸
Andrade LAF, Selikhova M, Lees AJ. Konstantin N. Tretiakoff in Brazil a historical perspective and discussion of his contribution to Brazilian neuroscience. Arq Neuropsiquiatr 2009;67:322-327.

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